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. 2020 Feb 10;20(1):63.
doi: 10.1186/s12887-020-1956-y.

Posterior only instrumented fusion provides incomplete curve control for early-onset scoliosis in type 1 neurofibromatosis

Siyi Cai  1 , Zhengyao Li  1 , Guixing Qiu  1 , Jianxiong Shen  1 , Hong Zhao  1 , Yu Zhao  1 , Yipeng Wang  1 , Jianguo Zhang  2
Affiliations

Posterior only instrumented fusion provides incomplete curve control for early-onset scoliosis in type 1 neurofibromatosis

Siyi Cai et al. BMC Pediatr. .

Abstract

Background: The mid-long term outcomes of posterior spinal fusion in pediatric neurofibromatosis type 1 (NF-1) patients are rarely reported, so does the effectiveness of itsorthopeidc maintenance function. This study aims to evaluate the mid-long term surgical outcomes of posterior only instrumented spinal fusion for early-onset scoliosis (EOS) in NF-1 patients.

Methods: A retrospective review was performed on a cohort of 10 NF-1 patients having EOS from 2008 to 2014 in our hospital, the age averaged at 7.8 years old when they underwent posterior only instrumented spinal fusion for their EOS. Both general clinical data and surgical specific data of the patients were collected and reviewed, and the dystrophic progression of EOS was evaluated during the follow-up.

Results: The average duration of follow-up was 54 months (24 to 88 months). All patients underwent posterior only instrumented spinal fusion at 1 stage. The primary curves of EOS were thoracic in 9 cases and 1 patient had lumbar scoliosis. Preoperative major curve was significantly corrected (from 66.1 to 31.1 degrees). However, the major curve deteriorated significantly to 40.1 degrees on average at the end of the follow-up. The T1-S1 distance increased 2.8 cm on average and kept increasing at a rate of 0.6 cm/year during the follow-up.

Conclusions: Posterior only fusion surgery was not a good option to treat the EOS in NF-1 patients despite the relatively short segments involvement in the disease. The maintenance of orthopedic effect after treatment was not satisfactory.

Keywords: Early-onset scoliosis; Long-term; Neurofibromatosis; Posterior fusion; Surgical outcome.

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Conflict of interest statement

The authors declare that there is no conflict of interest regarding the publication of this article, and all the authors and the institution are consented to publish.

The authors declare that there is no conflict of interest regarding the publication of this article,

Figures

Fig. 1
Fig. 1
Case 6: Radiographs of a 4-year-old patient with neurofibromatosis and a 60.9°right thoracic scoliosis, who received posterior only fusion operation from T7-T11. A.B. Preoperation. C.D. Postoperation. E.F. The adding on phenomenon (arrow) was obvious at the 34-month follow-up, which was attributed to at the growth of the anterior column of the fusion segments
Fig. 2
Fig. 2
Case 2: A 7-year-old patient with neurofibromatosis and a 70.0°left Thoracolumbar scoliosis, who received posterior only fusion operation from T9-L3. A.B. Preoperation. C.D. Postoperation. E.F. The patient had severe thoracic-lumbar kyphosis deterioration at the 5-year follow-up, and his rod on the convex side slipped from the distal screw track (arrow). The posterior fusion alone was insufficient to inhibit the growth of the fusion segments even using the pedicle screws
See this image and copyright information in PMC

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